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B
Brian C. Mansfield
national institutes of health (nih) - usa
36
H指数
223
论文数
4.1K
被引数
0
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43
发表时间
发表时间
IF
被引数
Persistent Activation of Renal Autophagy Contributes to Nephropathy in Murine Glycogen Storage Disease Type Ia
肾自噬的持续激活对小鼠糖原贮积症Ia型肾病的发生具有贡献作用。
JOURNAL OF INHERITED METABOLIC DISEASE
IF
3.8
2025-12-08
0
PRE
AI
Lee, Cheol; Pratap, Kunal; Gautam, Sudeep; Zhang, Lisa; Chen, Hung Dar; Loh, Yoke Peng; Mansfield, Brian C.; Chou, Janice Y.
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Novel murine model provides insights into early-onset of kidney disease in glycogen storage disease type Ib
新型小鼠模型为糖原贮积症Ib型早期肾病的发病机制提供了新的见解。
Molecular Genetics and Metabolism
IF
3.5
2025-10-10
0
PRE
AI
Kunal Pratap; Cheol Lee; Lisa Zhang; Hung Dar Chen; Irina Arnaoutova; Brian C. Mansfield; Janice Y. Chou
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The Wnt/β-catenin signaling pathway mediates renal fibrosis in glycogen storage disease type Ib nephropathy
Wnt/β-catenin信号通路介导糖原累积病Ib型肾病的肾纤维化
biochimica et biophysica acta molecular basis of disease
IF
0
2025-07-08
0
PRE
AI
Kunal Pratap; Cheol Lee; Lisa Zhang; Hung Dar Chen; Irina Arnaoutova; Brian C. Mansfield; Janice Y. Chou
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Liver-Directed Gene Therapy Mitigates Early Nephropathy in Murine Glycogen Storage Disease Type Ia
肝脏靶向基因疗法可减轻小鼠糖原贮积病Ia型的早期肾病
Journal of Inherited Metabolic Disease
IF
3.8
2025-05-30
0
OA
AI
Cheol Lee; Kunal Pratap; Lisa Zhang; Hung Dar Chen; Irina Arnaoutova; Matthew F. Starost; Brian C. Mansfield; Janice Y. Chou
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Base-editing corrects metabolic abnormalities in a humanized mouse model for glycogen storage disease type-Ia
NATURE COMMUNICATIONS
IF
15.7
2024-11-10
0
OA
AI
Arnaoutova, Irina; Aratyn-Schaus, Yvonne; Zhang, Lisa; Packer, Michael S.; Chen, Hung-Dar; Lee, Cheol; Gautam, Sudeep; Gregoire, Francine M.; Leboeuf, Dominique; Boule, Steven; Fernandez, Thomas P.; Huang, Victoria; Cheng, Lo-, I; Lung, Genesis; Bannister, Brianna; Decker, Jeremy; Leete, Thomas; Shuang, Lan S.; Bock, Caroline; Kothiyal, Prachi; Grayson, Phil; Mok, Ka W.; Quinn, Jeffrey J.; Young, Lauren; Barrera, Luis; Ciaramella, Giuseppe; Mansfield, Brian C.; Chou, Janice Y.
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Disease modeling and pharmacological rescue of autosomal dominant retinitis pigmentosa associated with RHO copy number variation
ELIFE
IF
0
2024-04-25
2
OA
AI
Kandoi, Sangeetha; Martinez, Cassandra; Chen, Kevin Xu; Mehine, Miika; Reddy, L. Vinod K.; Mansfield, Brian C.; Duncan, Jacque L.; Lamba, Deepak A.
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Eupatilin Improves Cilia Defects in Human CEP290 Ciliopathy Models
CELLS
IF
5.2
2023-06-07
12
OA
AI
Corral-Serrano, Julio C.; Sladen, Paul E.; Ottaviani, Daniele; Rezek, Olivia F.; Athanasiou, Dimitra; Jovanovic, Katarina; van der Spuy, Jacqueline; Mansfield, Brian C.; Cheetham, Michael E.
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Molecular mechanism underlying impaired hepatic autophagy in glycogen storage disease type Ib
HUMAN MOLECULAR GENETICS
IF
3.2
2022-08-12
7
OA
AI
Gautam, Sudeep; Zhang, Lisa; Lee, Cheol; Arnaoutova, Irina; Chen, Hung Dar; Resaz, Roberta; Eva, Alessandra; Mansfield, Brian C.; Chou, Janice Y.
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Correction of metabolic abnormalities in a mouse model of glycogen storage disease type Ia by CRISPR/Cas9-based gene editing
MOLECULAR THERAPY
IF
12
2021-04-01
19
OA
AI
Arnaoutova, Irina; Zhang, Lisa; Chen, Hung-Dar; Mansfield, Brian C.; Chou, Janice Y.
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The signaling pathways implicated in impairment of hepatic autophagy in glycogen storage disease type Ia
HUMAN MOLECULAR GENETICS
IF
3.2
2020-01-21
17
OA
AI
Gautam, Sudeep; Zhang, Lisa; Arnaoutova, Irina; Lee, Cheol; Mansfield, Brian C.; Chou, Janice Y.
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Liver-directed gene therapy for murine glycogen storage disease type Ib
HUMAN MOLECULAR GENETICS
IF
3.2
2017-08-21
18
OA
AI
Kwon, Joon Hyun; Lee, Young Mok; Cho, Jun-Ho; Kim, Goo-Young; Anduaga, Javier; Starost, Matthew F.; Mansfield, Brian C.; Chou, Janice Y.
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Downregulation of SIRT1 signaling underlies hepatic autophagy impairment in glycogen storage disease type Ia
PLOS GENETICS
IF
3.7
2017-05-30
57
OA
AI
Cho, Jun-Ho; Kim, Goo-Young; Pan, Chi-Jiunn; Anduaga, Javier; Choi, Eui-Ju; Mansfield, Brian C.; Chou, Janice Y.
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Downregulation of pathways implicated in liver inflammation and tumorigenesis of glycogen storage disease type Ia mice receiving gene therapy
HUMAN MOLECULAR GENETICS
IF
3.2
2017-03-13
23
OA
AI
Kim, Goo-Young; Kwon, Joon Hyun; Cho, Jun-Ho; Zhang, Lisa; Mansfield, Brian C.; Chou, Janice Y.
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Glycogen storage disease type Ia mice with less than 2% of normal hepatic glucose-6-phosphatase-α activity restored are at risk of developing hepatic tumors
MOLECULAR GENETICS AND METABOLISM
IF
3.5
2017-03-01
28
OA
AI
Kim, Goo-Young; Lee, Young Mok; Kwon, Joon Hyun; Cho, Jun -Ho; Pan, Chi-Jiunn; Starost, Matthew F.; Mansfield, Brian C.; Chou, Janice Y.
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Mice expressing reduced levels of hepatic glucose-6-phosphatase-α activity do not develop age-related insulin resistance or obesity
HUMAN MOLECULAR GENETICS
IF
3.2
2015-06-18
16
OA
AI
Kim, Goo-Young; Lee, Young Mok; Cho, Jun-Ho; Pan, Chi-Jiunn; Jun, Hyun Sik; Springer, Danielle A.; Mansfield, Brian C.; Chou, Janice Y.
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Functional analysis of mutations in a severe congenital neutropenia syndrome caused by glucose-6-phosphatase-β deficiency
MOLECULAR GENETICS AND METABOLISM
IF
3.5
2015-01-01
10
OA
AI
Lin, Su Ru; Pan, Chi-Jiunn; Mansfield, Brian C.; Chou, Janice Yang
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The upstream enhancer elements of the G6PC promoter are critical for optimal G6PC expression in murine glycogen storage disease type Ia
MOLECULAR GENETICS AND METABOLISM
IF
3.5
2013-11-01
22
OA
AI
Lee, Young Mok; Pan, Chi-Jiunn; Koeberl, Dwight D.; Mansfield, Brian C.; Chou, Janice Y.
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The SLC37 family of phosphate-linked sugar phosphate antiporters
磷酸连接的糖磷酸反转运蛋白的SLC37家族
MOLECULAR ASPECTS OF MEDICINE
IF
10.3
2013-04-01
27
OA
AI
Chou, Janice Y.; Jun, Hyun Sik; Mansfield, Brian C.
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Prevention of hepatocellular adenoma and correction of metabolic abnormalities in murine glycogen storage disease type Ia by gene therapy
HEPATOLOGY
IF
15.8
2012-11-01
65
OA
AI
Lee, Young Mok; Jun, Hyun Sik; Pan, Chi-Jiunn; Lin, Su Ru; Wilson, Lane H.; Mansfield, Brian C.; Chou, Janice Y.
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A Method for Monitoring and Controlling Reproducibility of Intensity Data in Complex Electrospray Mass Spectra: A Thermometer Ion-based Strategy
JOURNAL OF THE AMERICAN SOCIETY FOR MASS SPECTROMETRY
IF
2.7
2011-11-22
20
PRE
AI
Lecchi, Paolo; Zhao, Jinghua; Wiggins, Wesley S.; Chen, Tzong-Hao; Yip, Ping F.; Mansfield, Brian C.; Peltier, John M.
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研究方向
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合作期刊
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H 指数: 105 · 论文数: 620
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H 指数: 103 · 论文数: 445
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H 指数: 87 · 论文数: 475
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H 指数: 79 · 论文数: 248
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H 指数: 71 · 论文数: 483
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